[Show abstract][Hide abstract]ABSTRACT:
Behcet's disease is multisystemic vasculitis which affects vein and artery blood vessels. Intestinal perforation rarely occurs as clinic manifestation in as little as 1% of patients. The transverse colon is the most infrequent site of perforation. We presented a patient diagnosed with Behcet's disease who underwent both surgical and conversative treatment due to perforation of the colon.
A 34-year-old patient was admitted to the hospital with fever, aphthous ulcerations on oral mucosa and genitals and bilateral uveitis. On the basis of clinical symptoms and the International Criteria developed in 1990 Behcet's disease was diagnosed. During the next few days the patient developed erythema nodosum, diarrheic syndrome and acute abdominal symptoms due to perforation of the transverse colon. An emergent laparotomy was undertaken involving resection of a perforated segment of the colon, and bipolar colostomy on the left side of abdomen. Following the surgery the patient was treated except for antibiotics with three successive pulse doses of methylprednisolone (500 mg/daily) and cyclophosphamide (15 mg/kg). The treatment was continued by gradual decrease in the close of the corticosteroid (perorally) and by cyclophosphamide first with monthly doses (5 monthly pulse doses of 15 mg/kg cyclophosphoamide), and then with 3-month doses (totally 6 doses) up to totally 12 g.
The therapy with pulse doses of methylprednisolone combined with pulse doses of cyclophosphamide was very effective in the reported case with the complex clinical manifestations leading to resolution of gastrointenstinal, ocular and orogenital lesions.

[Show abstract][Hide abstract]ABSTRACT:
Behçet's disease (BD) is a vasculitic disorder of relapsing acute inflammation characterized by recurrent oral ulcers, genital ulcers, uveitis, and skin lesions. The disease also affects other organs, including joints, the nervous system, blood vessels, and gastrointestinal (GI) system may also be involved and the lower GI tract is the mostly involved part, leading to severe morbidity. The frequency of GI involvement in BD varies among different ethnic groups. Although 50-60% of Japanese patients have GI disease, these manifestations are rare in patients from Mediterranean countries. The gastrointestinal manifestations of BD usually appear 4.5-6 years after the onset of the oral ulcers. The intestinal lesions are usually resistant to medical treatment and recur after surgery. The elementary lesion is apthous ulcer. Deep, round or oval ulcers with a punched-out appearance tend to perforate easily, so that many patients require urgent operation.

01/2012; 2012:216254. DOI:10.1155/2012/216254

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doi: 10.1590/S1807-59322009001200016Kemal Karakaya,I Mustafa Comert,I Gamze NumanogluIIINTRODUCTIONBehcet’s Disease (BD) is a rare and lifelong disorder characterized by inflammation of blood vessels throughout the body.1 BD is much more prevalent in countries along the ancient silk road from eastern Mediterranean countries to Eastern Asia.1,2 Some environmental factors are proposed for the etiology.3 Since Behcet’s4 original description in 1937, many other manifestations have been added to the original triad. Diagnostic criteria for BD were defined by the international study group for BD in 1990.5 Although the etiology of BD is unknown, it is likely due to an autoimmune vasculitis in genetically predisposed individuals triggered by an infectious agent or other antigens.2,6 BD mostly affects children and young adults between the second and fourth decades of life7. Those affected before the age of 25 years (early onset) and males have been shown to have more severe disease symptoms.8 Treatment of BD is usually palliative and symptomatic. Antibiotic, antiviral, corticosteroid, immunosuppressive and cytotoxic agents are used for treatment according to the symptoms. Unlike neuro-Behcet’s, entero-Behcet’s causes intestinal perforation/bleeding and some vascular lesions.9-11 The syndrome usually runs a relatively benign course with exacerbations and remissions.Gastrointestinal involvement of BD is rare.9 Common gastrointestinal sites affected by BD other than the mouth are the ileocecal region and the colon. There are only a few studies reporting colonic perforations due to BD. In this article, we report a patient with BD who underwent an emergency operation for acute abdomen that developed secondary to multiple perforations of the transverse colon. The patient had a good response to surgical treatment followed by corticosteroids, immunosuppressant drugs and colchicine.CASE DESCRIPTIONA 21-year-old man was admitted to the emergency department of our hospital complaining of severe abdominal pain, nausea, poor appetite and fever. Medical history revealed that five years ago he was diagnosed with BD because of his recurrent, painful oral and genital ulcers and positive pathergy test. Colchicine treatment was started and he was followed in an outpatient clinic. He had arthro-synthesis due to left knee arthritis two years after the diagnosis of BD while he was under colchicine therapy. Since then, he suffered several attacks of arthritis, and corticosteroid and nonsteroidal anti-inflammatory drugs were added. The patient had no family history of BD. Physical examination revealed diffuse abdominal tenderness and rebound. Oral and genital examinations were normal. Abdominal ultrasonography revealed massive free fluid and thickness of the bowel wall.An emergent laparotomy was undertaken on the patient. The peritoneal cavity was filled with purulent fluid. Swelling of the mesentery and thickness of the bowel wall, especially on the right side of the abdomen, was remarkable. During exploration, three separate perforations on the antimesenteric side in a 12-cm segment of the right half of the transverse

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1232CLINICS 2009;64(12):1231-3Multiple perforations along the transverse colon as a rare presentation of intestinal behcet’s diseaseKarakaya K et al.colon were observed. The involved segment was resected. The cut edges were free of ulceration on gross appearance. A double-barrel colostomy was performed on the right upper quadrant of the abdomen. Surgical specimen showed three perforations on the antimesenteric side of the colon. The perforations were 20 mm, 7 mm and 3.5 mm in diameter with a sharp margin of ulcer, a round, punched out figure (Figure 1). Postoperative course was uneventful. The patient was discharged on the fifth postoperative day. Histopathological analysis of the resected specimens demonstrated multiple round and ovoid mucosal ulcers with sharp margins, measuring up to 2 cm in maximal diameter. Many of these ulcers were penetrated or perforated and surrounded by extensive edema. Small venules and small arterioles in the submucosa and muscular layer of the entire colon segment were surrounded by extensive edema and diffuse mononuclear inflammatory cells (Figure 2).Azathioprim was added to the patient’s therapy. Endoscopic control of the upper and lower gastrointestinal tract revealed no ulceration three months after the operation. The patient’s colostomy was closed four months after the first operation under a semi-addisonian protocol. The postoperative period was uneventful, and the patient was discharged on the seventh postoperative day. He was clinically well during the six-month follow-up period after the operation while taking colchicine and azathioprim.DISCUSSIONDiagnosis of BD should be based on clinical criteria because there is no pathognomonic laboratory test to detect it.5 BD may be seen at any level of the gastrointestinal tract. A common gastrointestinal site other than the mouth is the ileocecal region.12 Gastrointestinal symptoms related to BD are abdominal pain, nausea and vomiting. Some symptoms present in emergency conditions, such as intestinal perforation or bleeding.10,13 Intestinal lesions are located on the antimesenteric side (Figure 1). Inflammatory bowel diseases should be kept in mind in the differential diagnosis of intestinal BD. The ulcers in intestinal BD may be aphtous or deep and round with a punched-out appearance. Although International Study Group criteria for BD accurately distinguish between BD and Crohn’s Disease,14 there are some common features. Like Crohn’s disease, BD manifests as discrete intestinal ulcers and discontinuous bowel involvement. Both of the diseases share extraintestinal manifestations, such as arthritis and uveitis. Rectal sparing is common for both. Longitudinal ulcers are rare in intestinal BD, but common in inflammatory bowel diseases. The ulcers may cause penetration, perforation or bleeding. Multiple ulcers are generally seen at multiple sites and may resolve with medical therapy. Our patient had multiple ulcers that led to intestinal perforation in the transverse colon. The remaining intestines were considered normal in gross appearance at laparotomy. A wide range of hallmarks were apparent in this case suggesting that the patient had BD, including changes of the non-ulcerative mucosa, venulitis on the order of vasculitis, characteristics of ulcers, absence of lymphoid aggregates and granulomas in the bowel. There was no ulcerative lesion determined by lower and upper GI endoscopy performed three months after surgery. It is also possible that intestinal ulcers might have healed with the corticosteroid and immunosuppressant agents added to the patient’s therapy before endoscopic evaluation. Treatment of BD is largely empirical, and a multidisciplinary approach is preferred. A multidisciplinary team should involve specialists in dermatology, rheumatology, ophthalmology, gastroenterology, immunology and others. Corticosteroids, immuno-suppressants, and other agents such as colchicine and interferon are used for the treatment of BD.Mesalazine and anti-tumor necrosis factors such as infliximab and thalidomide have been used for intestinal BD on a limited basis.15 The effectiveness of therapy on Figure 1 - Thick arrows indicate ulcers and thin arrows indicate macroscopic mucosal erosionsFigure 2 - (2A) Congestion, exudate and ulcer base with inflammation (H&E x10). (2B) Perforation along whole thickness of the bowel wall with exuda and erosion (H&Ex5)